OBJECTIVE: To assess the diagnostic standards and treatment of severe Budd-Chiari syndrome (BCS). METHODS: The clinical data of 126 patients with severe BCS treated from November 1994 to June 2001 at our hospital were retrospectively analyzed. Percutaneous transhepatic recanalization and dilation and/or stent placement of the main hepatic vein was performed in 10 patients. Mesocaval C type shunt with artificial graft was performed in 68 patients, splenojugular shunt in 33, mesojuglar shunt in 1, and mesocaval shunt or improved splenopneumopexy after percutaneous intraluminal angioplasty and stent placement of the inferior vena cava in 14. RESULT: Six patients died during perioperation. In 120 patients followed up for 6 months to 7 years, 89 had excellent results and 31 good results. CONCLUSIONS: Diagnostic standards of severe BCS are suggested. Proper treatment should be used according to the pathological changes of the inferior vena cava and main hepatic veins.